Progression of Aortic Dilation in Children With a Functionally Normal Bicuspid Aortic Valve

doi:10.1016/j.amjcard.2006.04.022

The American Journal of Cardiology

Volume 98, Issue 6, 15 September 2006, Pages 828-830

https://doi.org/10.1016/j.amjcard.2006.04.022 Get rights and content

Adults with bicuspid aortic valves (BAVs) are at risk for progressive aortic dilation independent of valve function. The evolution of aortic dilation in children with functionally normal BAVs has not been studied. In this study, ascending aortic diameters were assessed in a group of children with functionally normal BAVs (peak gradient ≤16 mm Hg) to determine whether progressive dilation occurs during childhood. A cohort of 101 patients and 97 controls were studied with echocardiography. There were no differences in mean age (9.0 ± 4.8 vs 8.7 ± 6.1 years, p = 0.7) or body surface area (1.1 ± 0.4 vs 1.1 ± 0.5 m², p = 0.9). Patients had significantly greater aortic dimensions than controls in all measured regions of the aorta, with the most striking discrepancy in the ascending aorta (2.3 ± 0.6 vs 1.8 ± 0.5 cm, p <0.0001). The size discrepancy was present from birth and persisted throughout childhood. Repeated measurements were performed in a subgroup of 28 patients and 25 controls. Patients had significantly greater increases in ascending aortic dimension than controls per year of follow-up (1.2 ± 0.08 vs 0.6 ± 0.08 mm/year, p <0.0001). In conclusion, BAV is complicated by progressive aortic dilation beginning in childhood.

Section snippets

Methods and Results

An institutional review board–approved echocardiographic and clinical analysis of children with isolated BAVs and healthy controls was undertaken. Patients with isolated BAVs were those with no greater than trivial aortic insufficiency and peak transvalvar aortic gradients of ≤16 mm Hg. Our aim was to determine whether a functionally normal BAV is a benign lesion or whether such patients require more frequent follow-up because of aortic disease progression early in life. Patients and controls

Discussion

Although aneurysmal aortic dilation has been documented in adults with isolated BAVs,⁵ this study supports the postulate that BAV is a disease of the entire aorta, with an associated aortopathy present from birth.⁶ In the absence of even minor hemodynamic alterations, we have shown aortic dilation to be present and progressive throughout childhood in patients with BAVs.

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Cited by (104)

Left ventricular strain in pediatric patients with bicuspid aortic valves and aortopathy
2023, Progress in Pediatric Cardiology
Bicuspid aortic valve is the most common congenital cardiac defect. Left ventricular strain has been shown to be lower in adults with bicuspid aortic valve and preserved ejection fraction. Additionally, left ventricular strain has been independently associated with aortic valve replacement and all-cause mortality in adults. In pediatric patients, left ventricular strain in patients with bicuspid aortic valve with and without aortopathy has not been evaluated.
We aimed to evaluate the effect of clinical aortopathy on left ventricular strain in pediatric patients with well-functioning bicuspid aortic valve compared to normal controls.
This is a single-center retrospective study in patients <21 years of age with normal ejection fraction and no prior cardiac interventions. Patients were divided into three groups: 1) healthy controls with normal transthoracic echocardiograms 2) well-functioning BAV without clinical aortopathy 3) well-functioning BAV with isolated clinical aortopathy. TTE images were analyzed to obtain left ventricle global longitudinal strain and left ventricle global circumferential strain, respectively. Strain values were compared across the three groups.
The mean values and 95 % confidence intervals (95%CIs) for LVGLS were −23.1 (−24.3, −22.0), −22.5 (−23.8, −21.1), and −21.2 (−22.5, −20.0) for groups 1, 2 and 3 respectively. There was a statistically significant difference in Left ventricular global longitudinal strain in group 3 compared to group 1(p = 0.033). There was no significant difference in global circumferential strain between the three groups.
Our study demonstrates lower left ventricular strain in patients with a well-functioning bicuspid aortic valve and clinical aortopathy compared to controls. This is suggestive of an early effect on the left ventricular myocardium caused by a change in loading conditions from alteration in aortic elasticity. Left ventricular strain is a potential early marker for this and can be utilized for further correlation with clinical outcomes.
New England Congenital Cardiology Association Bicuspid Aortopathy Registry (NECCA BAR): A regional preventive cardiovascular care collaboration
2022, Progress in Pediatric Cardiology
Bicuspid aortic valve is the most common congenital heart defect, and its association with proximal aortic dilation (aortopathy) increases risk for aortic aneurysm and dissection in adults. Maximal aortic root and ascending aortic dimensions are predictors of adverse cardiovascular outcomes. Recent single center data demonstrate medical therapy with beta or angiotensin II receptor blockers tempers aortic growth rates. Although randomized clinical trials may not be feasible, larger population studies would better inform bicuspid aortic valve preventive cardiovascular care.
Improve regional pediatric bicuspid aortopathy collaboration with standardized medical management guidelines and patient care registry.
The New England Congenital Cardiology Association (NECCA) developed consensus-based prophylactic treatment guidelines for bicuspid aortopathy and designed a regional clinical registry (NECCA BAR) to study cardiovascular practice variation and short-term outcomes in subjects with moderate to severe bicuspid aortopathy. Individuals 6 months to 25 years of age with bicuspid aortic valve (all variants) and body surface area-adjusted aortic root or ascending aorta z score ≥ 4.0 SD, and/or absolute dimension ≥4.0 cm, are eligible for inclusion. Standardized bicuspid aortopathy medical management guidelines were established among New England congenital cardiology practices. The primary aims are to compare cardiovascular practice variation by guideline adherence (medical therapy and athletic participation), and evaluate medication effectiveness by comparing rates of annual change in aortic root and ascending aorta dimensions/z scores over 3 years. Additional endpoints include aortic valve disease progression (stenosis/regurgitation), incidence of aortic dissection, surgery, death, and incidence of adverse drug events.
Recruitment in progress; data analysis pending.
We describe the development of regional pediatric clinical care guidelines and a patient care registry designed to refine medical management of bicuspid aortopathy.
Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve
2022, Canadian Journal of Cardiology
Bicuspid aortic valve (BAV) is the most common congenital heart disease, often associated with valve dysfunction, coarctation of the aorta, and ascending aorta dilatation. Aortic dilatation might result from abnormal regional hemodynamics or inherent vascular disease. Vascular function in pediatric BAV remains poorly characterized.
A cross-sectional study was performed to evaluate vascular function in 142 children with BAV aged 7-18 years compared with healthy control children. Echocardiography was performed to assess aortic dimensions, BAV function, and vascular function (aortic arch pulse wave velocity [PWV]), carotid intima media thickness, and aortic stiffness and distensibility). Carotid-femoral and carotid-radial PWV were assessed using tonometry. Vascular function was compared for 4 patient groups stratified according to aortic dilatation and a history of coarctation of the aorta. Multivariate regression analysis was performed to determine predictors of aortic dilatation.
Children with BAV had stiffer and less distensible ascending aortas with higher aortic arch PWV compared with control children. Carotid-femoral and carotid-radial PWV were not increased in patients with BAV, and the vascular assessment of the abdominal aorta was unremarkable. Multivariate regression revealed that aortic arch PWV was the only vascular function parameter that was associated with aortic dilatation.
Children with BAV have differences in vascular function that are confined to their proximal aorta, even in normal functioning BAV. The observed differences in vascular function are likely multifactorial, with contributions from abnormal regional flow and a potential localized primary aortopathy.
La bicuspidie valvulaire aortique (BVA) est la cardiopathie congénitale la plus fréquente. Elle est souvent associée à une dysfonction valvulaire, à une coarctation de l’aorte et à une dilatation de l’aorte ascendante. La dilatation aortique peut résulter d’une anomalie de l’hémodynamique locale ou d’une vasculopathie intrinsèque. La fonction vasculaire des enfants atteints de BVA demeure mal caractérisée.
Une étude transversale a été menée afin de comparer la fonction vasculaire de 142 enfants atteints de BVA âgés de 7 à 18 ans à celle d’enfants du groupe témoin en bonne santé. Les sujets ont subi une échocardiographie afin d’évaluer les caractéristiques suivantes : dimensions de l’aorte, fonction de la valvule aortique et fonction vasculaire (vélocité de pouls [VOP] de l’arc de l’aorte, épaisseur de l’intima-média de la carotide ainsi que rigidité et capacité de dilatation de l’aorte). La VOP carotido-fémorale et carotido-radiale ont été évaluées par tonométrie. La fonction vasculaire a été comparée chez les quatre groupes de patients stratifiés en fonction de la dilatation aortique et des antécédents de coarctation de l’aorte. Une analyse de régression multivariée a été effectuée afin de déterminer les prédicteurs de la dilatation de l’aorte.
L’aorte ascendante des enfants atteints de BVA était plus rigide et moins distensible que celle des enfants du groupe témoin, et la VOP de leur arc aortique était plus élevée. Les VOP carotido-fémorale et carotido-radiale n’étaient pas plus élevées, et l’évaluation vasculaire de leur aorte abdominale n’a rien révélé de particulier. L’analyse de régression multivariée a indiqué que la VOP de l’arc aortique était le seul paramètre de la fonction vasculaire associé à une dilatation aortique.
Les enfants atteints de BVA présentent des différences de fonction vasculaire qui se limitent à la portion proximale de l’aorte, même si la valve aortique bicuspide fonctionne normalement. La cause de ces différences de fonction vasculaire est probablement multifactorielle, s’expliquant entre autres par une anomalie du flux régional et une possible aortopathie primaire localisée.
Effect of Losartan or Atenolol on Children and Young Adults With Bicuspid Aortic Valve and Dilated Aorta
2021, American Journal of Cardiology
Citation Excerpt :
Our study results suggest that both angiotensin II receptor blockers and beta blockers reduce the rate of growth of the proximal aorta in patients with moderate to severe BAV-associated aortopathy. Before initiation of medical prophylaxis, AoRt and AsAo z-scores increased in both treatment groups, consistent with previous natural history studies showing progressive aortic dilation during childhood reporting z-score slopes ranging from 0.2 to 0.4SD/yr.13,15,16 In the losartan group, treatment was associated with a reduction in AoRt z-score over time, and zero change (stabilization) in AsAo z-score.
Bicuspid aortic valve aortopathy is defined by dilation of the aortic root (AoRt) and/or ascending aorta (AsAo), and increases risk for aortic aneurysm and dissection. The effects of medical prophylaxis on aortic growth rates in moderate to severe bicuspid aortopathy have not yet been evaluated. This was a single-center retrospective study of young patients (1 day to 29 years) with bicuspid aortopathy (AoRt or AsAo z-score ≥ 4 SD, or absolute dimension ≥ 4 cm), treated with either losartan or atenolol. Maximal diameters and BSA-adjusted z-scores obtained from serial echocardiograms were utilized in a mixed linear effects regression model. The primary outcome was the annual rate of change in AoRt and AsAo z-scores during treatment, compared with before treatment. The mean ages (years) at treatment initiation were 14.2 ± 5.1 (losartan; n = 27) and 15.2 ± 4.9 (atenolol; n = 18). Median treatment duration (years) was 3.1 (IQR 2.4, 6.0) for losartan, and 3.7 (IQR 1.4, 6.6) for atenolol. Treatment was associated with decreases in AoRt and AsAo z-scores (SD/year), for both losartan and atenolol (pre- vs post-treatment): losartan/AoRt: +0.06 ± 0.02 vs -0.14 ± 0.03, p < 0.001; losartan/AsAo: +0.20 ± 0.03 vs -0.09 ± 0.05, p < 0.001; atenolol/AoRt: +0.07 ± 0.03 vs -0.02 ± 0.04, p = 0.04; atenolol/AsAo: +0.21 ± 0.04 vs -0.06 ± 0.06, p < 0.001. Treatment was also associated with decreases in absolute growth rates (cm/year) for all comparisons (p ≤ 0.02). Medical prophylaxis reduced proximal aortic growth rates in young patients with at least moderate and progressive bicuspid aortopathy.
Should all patients with aortic aneurysm and bicuspid aortic valve also undergo hemiarch?
2021, JTCVS Open
Aortopathy in Congenital Heart Disease
2020, Cardiology Clinics

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Congenital heart diseaseProgression of Aortic Dilation in Children With a Functionally Normal Bicuspid Aortic Valve

Section snippets

Methods and Results

Discussion

Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction

Am J Cardiol

Frequency of aortic root dilation in children with a bicuspid aortic valve

Am J Cardiol

Echocardiographic assessment of aortic root dimensions in normal children based on measurement of a new ratio of aortic size independent of growth

Am J Cardiol

Congenital heart disease
Progression of Aortic Dilation in Children With a Functionally Normal Bicuspid Aortic Valve